Vulval Syringoma: First report of a case in an Egyptian female

Abstract

A 23-year-old married woman presented with a history of multiple, pinhead-sized vulval skin lesions since nine months. The patient noticed no exacerbation of the lesions during menstruation or summer season. There was no history of similar lesions elsewhere or in among the family members. She denied any extramarital relationships. She had been treated with topical steroid preparations in the past without any relief. Serology for VDRL and HIV was negative. Abdominal ultrasonography revealed no organomegaly. On examination, there were multiple, discrete, bilateral and symmetrical soft skin-colored papules with smooth surface measuring 2-5 mm in diameter present over the labia majora [Figure 1a and b]. Figure 1a,b Skin colored bilaterally symmetrical papules over the vulva Per speculum examination revealed no similar vaginal or cervical lesions. Systemic examination was normal. A clinical differential diagnosis of syringomas, Fox-Fordyce's disease, milia, epidermal cysts, steatocystoma multiplex and genital warts was considered. Histopathology of one of the lesions revealed numerous cystic ducts lined by two rows of epithelial cells embedded in a fibrocollagenus stroma [Figure 2a]. Lumen of the ducts contained amorphous debris. Some of the ducts showed small comma like tails of epithelial cells (tadpole appearance) in the papillary and reticular dermis [Figure 2b]. These observations were consistent with the clinical diagnosis of syringoma. Figure 2a Numerous cystic ducts lined by two rows of epithelial cells embedded in a fibrocollagenous stroma (H and E, ×20) Figure 2b Lumen of ducts containing amorphous debris. Some of the ducts show small comma-like tails of epithelial cells (tadpole appearance) (H and E, ×40) Syringoma is a benign neoplasm that arises from the intraepidermal eccrine sweat duct. Its name was derived from the Greek word syrinx, which means pipe or tube.[1] Syringoma was first described in 1872 by Kaposi and Biesiadeki as lymphangioma tuberosum multiplex.[2] The prevalence of this tumor may be greater than previously reported due to the fact that many are asymptomatic and are unrecognized by both patients and clinicians. Vulvar syringomas (VS) have commonly been described in association with extragenital lesions.[3] Our case showed only vulval lesions with clinical and histological features of syringoma. The main reason for treatment is cosmetic disfigurement, the treatment options being dermabrasion, cryotherapy, electro-cauterisation, CO 2laser and full-thickness grafting.[4] The aim of reporting this case is to highlight a rare genital lesion which, though benign, is embarrassing to the patient. VS are under reported as they may not be recognized over the genitalia in case they are asymptomatic. VS must be considered in the differential diagnosis of all papular lesions over the vulva.